What are the four structural heart anomalies that make up the tetralogy of Fallot select all that apply quizlet?

TEXTBOOKS Buyse ML, ed. Birth Defects Encyclopedia. Dover, MA: Blackwell Scientific Publications; For: The Center for Birth Defects Information Services Inc; 1990:1308.

Behrman RE, ed. Nelson Textbook of Pediatrics, 15th ed. Philadelphia, PA: W.B. Saunders Company; 1996:1311-15.

Braunwald E, ed. Heart Disease. A Textbook of Cardiovascular Medicine. 3rd ed. Philadelphia, PA: W. B. Saunders Company; 1988:946-8, 988-91.

JOURNAL ARTICLES
Therrien J, et al. A pilot study of exercise training in adult patients with repaired tetralogy of Fallot. Can J Cardiol. 2003;19:685-89.

Walker WT, et al. Quality of life after repair of tetralogy of Fallot. Cardiol Young. 2002;12:549-53.

Mulder TJ, et al. A multicenter analysis of the choice of initial surgical procedure in tetralogy of Fallot. Pediatr Cardiol. 2002;23:580-86.

Eldadah ZA, et al. Familial tetralogy of Fallot caused by mutation in the jagged1 gene. Hum Mol Genet. 2001;10:163-69.

Goldmuntz E, et al. NKX2.5 mutations in patients with tetralogy of Fallot. Circulation. 2001;104:2565-68.

Digilio MC, et al. Recurrence risk figures for isolated tetralogy of Fallot after screening 22q11 microdeletion. J Med Genet. 1997;34:188-90.

Santoro G, et al. Echocardiographically guided repair of tetralogy of Fallot. Am J Cardiol. 1994;73:808-11.

Sousa Uva M, et al. Surgery for tetralogy of Fallot at less than six months of age. J Thorac Cardiovasc Surg. 1994;107:1291-300.

Yamagishi M, et al. Outflow reconstruction of tetralogy of Fallot using a Gore-Tex valve. Ann Thorac Surg. 1993;56:1416-17.

Heinemann MK, et al. Preoperative management of neonatal tetralogy of Fallot with absent pulmonary valve syndrome. Thorac Surg. 1993;55:172-74.

Murphy JG, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. 1993;329:593- 99.

Warnes CA., Tetralogy of Fallot and pulmonary atresia/ventricular septal defect. Cardiol Clin. 1993;11:643-50.

INTERNET
Bhimji, S and Mancini MC. Tetralogy of Fallot. Medscape. Last Updated December 06, 2015. http://emedicine.medscape.com/article/2035949-overview. Accessed August 18, 2016.

McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No:187500; Last Update 9/30/2013.. http://omim.org/entry/187500. Accessed August 18, 2016.

Weinrauch LA. Tetralogy of Fallot. MedlinePlus. Last Update 10/22/2015. http://www.nlm.nih.gov/medlineplus/ency/article/001567.htm. Accessed August 18, 2016.
What is Tetralogy of Fallot?. National Heart, Lung & Blood Institute. (NHLBI). Last Updated July 1, 2011. http://www.nhlbi.nih.gov/health/health-topics/topics/tof. Accessed August 18, 2016.

Previously a routine technique, making a large incision in the right ventricle (ventriculotomy) and using a large transannular patch to repair TOF, have been abandoned. Extensive incisions, patches, and suturing (stitches) lead to the formation of scar tissue in the right ventricle, which is associated with weakness of heart muscle and heart conduction (electrical) system issues. Currently, the goal of surgical techniques is to minimize the extent incision (or cutting) of right ventricle and to maintain the integrity and competence of the pulmonary valve.

Despite surgical procedures to augment blood flow to the lungs as well as complete repair operation, some patients may develop long-term complications:

Right Ventricular Hypertrophy (RVH)
Obstruction (RVOT or pulmonary stenosis) forces the right ventricle to work harder to pump blood through the narrowed area. Over time, the muscle of the right ventricle may become thick, bulky, and not pump efficiently.

Right Ventricular Enlargement (RVE)
More commonly, RVE results from chronic leaking of blood from the pulmonary valve, back into the right ventricle. This extra blood volume causes enlargement (stretching) of the right ventricle, resulting in a boggy ventricle that does not pump efficiently. This may lead to heart failure, abnormal heart rhythms, and the tricuspid valve leaflets may lose their ability to close properly, resulting in tricuspid valve leaking as well.

 

Right Ventricular Failure
Chronic high-pressure and volume loading on the right ventricle eventually interferes with its ability to pump adequately. Further deterioration in pump function results in right ventricular failure. Symptoms associated with right ventricular failure include exercise intolerance, visible pulsations in the jugular veins, enlargement of the liver (sometimes associated with a feeling full after a small amount of food intake), weight gain due to fluid accumulation, and swelling in the hands, feet or legs. 

Ventricular Arrhythmias
The combination of RVH, RVE, right ventricular dysfunction and previous surgical incisions can all place TOF patients at substantially increased risk of heart rhythm problems. Patients can develop slowing of the heart’s conduction (electrical) system abnormalities called right bundle branch block, abnormal heart rhythms from the upper cardiac chambers (atrial arrhythmias) called atrial fibrillation or atrial flutter, or life-threatening heart rhythms from the bottom cardiac chambers (ventricular arrhythmias) called ventricular tachycardia or fibrillation. Therefore, it is very important to routinely monitor for heart rhythm problems. Information from multiple sources indicates that the risk for sudden cardiac death in patients with repaired TOF is 100 times greater than in the general population.

Aortic Regurgitation and Aortic Dilatation
Aortic valve regurgitation (leaking) occurs due to damage of the aortic valve with VSD repair. The aortic tissue is thought to be abnormal in patients with TOF and may account for aortic enlargement/aneurysm.

Branch Pulmonary Artery Stenosis
Either as part of TOF anatomy from birth but usually as a result of surgical shunts that were placed, the right and left pulmonary arteries may become narrowed reducing the blood flow to the right or left lung. This also puts excessive pressure on the right ventricle.